Long-term clinical and radiological results for fat graft obliteration in subtotal petrosectomy and cochlear implant surgery: a retrospective clinical study.

PURPOSE: The study aimed to evaluate the long-term clinical, radiological, and functional results for subtotal petrosectomy and cochlear implant surgery with closure of the external auditory canal and fat obliteration. METHODS: We retrospectively included all consecutive cases of simultaneous subtotal petrosectomy and cochlear implant surgery performed at a tertiary referral center between 2009 and 2016 using the same surgical technique. All patients underwent postoperative high-resolution computed tomography (HRCT) and annual audiological assessments. A 5-year minimum clinical, radiological, and audiological follow-up was performed. The early and late postoperative results were compared. The main outcome measures were complications, postauricular retraction, fat graft reabsorption, and audiological outcomes. RESULTS: Twenty-nine procedures performed in 23 patients (six bilateral) met the inclusion criteria. The mean age of the patients was 67 ± 13.4 years and mean follow-up duration was 7.5 ± 2 years. At follow-up, postauricular retraction was detected in 24 cases (82.8%), including five cases (17.1%) with subcutaneous protrusion of implant and array. Fat graft volume was significantly reduced at late-HRCT in terms of maximum diameter (2.24 ± 1.0 cm vs 3.69 ± 0.7 cm; p < 0.0005) and surface area (1.88 ± 1.2 vs 4.24 ± 1.6 cm2, p < 0.0005). Six patients had extracochlear electrodes at late-HRCT (3/6 had an increased number of extracochlear electrodes), with a lowering of this group's performance of - 15% (p < 0.005) in the follow-up speech comprehension test. CONCLUSIONS: Subtotal petrosectomy with cochlear implantation is an effective long-term technique in selected cases. Fat grafts showed significant reabsorption at long-term follow-up with reaeration of the middle ear spaces. Prolonged clinical and radiological follow-up is recommended for monitoring implant performances and late complications.

Pseudo-continuous arterial spin labelling shows high diagnostic performance in the detection of postoperative residual lesion in hyper-vascularised adult brain tumours.

OBJECTIVES: Our aim was to evaluate the contribution of pseudo-continuous arterial spin labelling (pCASL) in the detection of a postoperative residual lesion in adult brain tumours. METHODS: Seventy-five patients were prospectively included. Following the results of preoperative DSC-PWI assessment, intra-axial lesions, including high-grade gliomas (n = 43) and certain metastases (n = 14), were classified as hyper-vascular (HV+ group, n = 57); other lesions, including low-grade gliomas and certain metastases, were classified as non-hyper-vascular (HV- group, n = 18). To confirm the absence/presence of a residual lesion or disease progression, postoperative MRI including pCASL sequence and follow-up-MRI were performed within 72 h and 1-6 months after the resection, respectively. Two raters evaluated the images. Mean and maximal ASL cerebral blood flow (CBF) values were measured in the perioperative region and normalised to the contralateral tissue. The pCASL-CBF maps and post-contrast T1WI were visually assessed for residual lesion. Quantitative data were analysed with unpaired Student t and Mann-Whitney U tests and the visual diagnostic performance with the McNemar test. RESULTS: In the HV+ group, the mean normalised CBF was 1.97 ± 0.59 and 0.97 ± 0.29 (p < 0.0001, AUC = 0.964, cut-off = 1.27) for patients with or without residual tumours, respectively. The mean normalised CBF was not discriminative for assessing residual tumours in the HV- group (p = 0.454). Visual CBF evaluation allowed 92.98% patients belonging to the HV+ group to be correctly classified (sensitivity 93.02%, specificity 92.86%, p < 0.001). Visual evaluation was correlated with contrast enhancement evaluation and with the mean normalised CBF values (r = 0.505, p < 0.0001 and 0.838, p < 0.0001, respectively). CONCLUSION: Qualitative and quantitative ASL evaluation shows high diagnostic performance in postoperative assessment of hyper-perfused tumours. In this case, postoperative pCASL may be useful, especially if contrast injection cannot be performed or when contrast enhancement is doubtful. KEY POINTS: • Evaluation of postoperative residual lesion in the case of brain tumours is an imaging challenge. • This prospective monocentric study showed that increased normalised cerebral blood flow assessed by pseudo-continuous arterial spin labelling (pCASL) correlates well with the presence of a residual tumour in the case of hyper-vascular tumour diagnosed on preoperative MRI. • Qualitative and quantitative pCASL is an informative sequence for hyper-vascular residual tumour, especially if acquired more than 48 h after brain tumour surgery, when contrast enhancement can give ambiguous results due to blood-brain barrier disruption.

Specificities of arterial spin labeling (ASL) abnormalities in acute seizure.

PURPOSE: Arterial spin labeling (ASL) is a non-invasive tool measuring cerebral blood flow (CBF) and is useful to assess acute neurological deficit. While acute stroke presents as hypoperfused vascular territory, epileptic activity causes cortical hyperperfusion. Other neurological conditions exhibit hyperperfusion, like migraine or secondary "luxury perfusion" in strokes. Our objectives were to evaluate the usefulness and potential specificities of ASL in acute seizure and correlate it with electroencephalogram. MATERIALS AND METHODS: Amongst a cohort of patients with neurological deficit, addressed for suspicion of stroke, we retrospectively reviewed 25 consecutive patients with seizures who underwent magnetic resonance imaging (MRI) with ASL and electroencephalography (EEG). We compared them with a control group of patients with migraine and stroke secondary re-perfusion, exhibiting ASL hyperperfusion. RESULTS: Lateralized cortical hyperperfusion (high relative CBF) was observed in all patients. Good topographic correlation with EEG was found in 18 patients (72%). Eight (32%) had hyperperfusion of ipsilateral pulvinar, 5 (20%) had hyperperfused contralateral cerebellar hemisphere, 16 (64%) presented diffusion abnormalities and 20 (80%) had underlying epileptogenic lesions. Pulvinar hyperperfusion was not observed in the control group, nor were diffusion abnormalities in migrainous patients. Contralateral cerebellar hyperperfusion was observed in two migrainous patient, without associated pulvinar activation, whereas all patients with cerebellar hyperperfusion in the study group had associated pulvinar activation. CONCLUSIONS: Elevated CBF can be observed in the epileptogenic zone, ipsilateral pulvinar and contralateral cerebellum (diaschisis) in seizure. These abnormalities seem specific when compared with other causes of hyperperfusion. Arterial spin labeling can be highly effective in the differential diagnosis of strokes.

Neuronal injuries evidenced by transient cortical magnetic resonance enhancement in hemiplegic migraine: A case report.

BACKGROUND: Magnetic resonance imaging abnormalities in hemiplegic migraine have been described previously but were limited to a cortical thickening and biphasic alternation of hypoperfusion and hyperperfusion. Our report reveals possible blood-brain barrier disruption during migraine. CASE: We present the first demonstrated case of regressive diffuse hemispheric cortical enhancement in sporadic hemiplegic migraine, with histological correlation revealing neuronal lesions similar to ischemic lesions. This is probably due to the severity of the attack as indicated by the left hemiplegia and transient altered consciousness in our 43-year-old male patient. CONCLUSION: Cortical contrast enhancement on 3D T1 images may suggest migraine severity and be predictive of neuronal loss.

Bioactive glass granules for mastoid and epitympanic surgical obliteration: CT and MRI appearance.

PURPOSE: To evaluate the appearance of mastoid and epitympanic obliteration using S53P4 bioactive glass (BG) granules in high-resolution computed tomography (HRCT) and MRI. MATERIALS AND METHODS: Patients undergoing mastoid and epitympanic obliteration between May 2013 and December 2015 were prospectively included in an uncontrolled clinical study. All patients underwent a temporal HRCT scan 1 year after surgery, aimed at evaluating the attenuation, homogeneity, and osseointegration of the BG granules, as well as the ventilation of the middle ear and the volume of the obliterated paratympanic spaces. If a cholesteatoma was found during surgery, additional MRI, including at least pre- and post-contrast T1-weighted, T2-weighted, and axial non-echo-planar diffusion-weighted (DW) sequences, was performed 1 year after surgery, to study the normal signal of the BG granules and the presence of residual cholesteatoma and/or other temporal bone pathologies. RESULTS: Seventy cases were included. On 1-year HRCT, the mean attenuation of the BG granules was 888.34 ± 166.10 HU. The obliteration was found to be mostly homogeneous with partial osseointegration. The appearance of the BG granules having a low-intensity signal in T2-weighted imaging and DW MRI was always different from the appearance of cholesteatoma. A longer follow-up has shown no attenuation or signal modification of the BG granules compared with the 1-year imaging. CONCLUSION: Radiological follow-up of patients operated on with mastoid and epitympanic obliteration using BG granules is effective using both HRCT and MRI. A cholesteatoma and/or other potential complications could easily be detected due to the specific radiological appearance of the BG granules. KEY POINTS: • The appearance of mastoid and epitympanic obliteration by S53P4 bioactive glass (BG) granules on high-resolution computed tomography (HRCT) scans was homogeneous with an attenuation significantly higher than the attenuation of cholesteatoma and lower than mastoid bone attenuation. • The granules have a low-intensity signal on non-echo-planar diffusion-weighted sequences and on T2-weighted images and present contrast enhancement allowing the differential diagnosis with cholesteatoma and effective for the detection of other underlying temporal bone pathologies. • The volume and radiological appearance of the obliteration appear to be stable with time.

Muscular MRI-based algorithm to differentiate inherited myopathies presenting with spinal rigidity.

OBJECTIVES: Inherited myopathies are major causes of muscle atrophy and are often characterized by rigid spine syndrome, a clinical feature designating patients with early spinal contractures. We aim to present a decision algorithm based on muscular whole body magnetic resonance imaging (mWB-MRI) as a unique tool to orientate the diagnosis of each inherited myopathy long before the genetically confirmed diagnosis. METHODS: This multicentre retrospective study enrolled 79 patients from referral centres in France, Brazil and Chile. The patients underwent 1.5-T or 3-T mWB-MRI. The protocol comprised STIR and T1 sequences in axial and coronal planes, from head to toe. All images were analyzed manually by multiple raters. Fatty muscle replacement was evaluated on mWB-MRI using both the Mercuri scale and statistical comparison based on the percentage of affected muscle. RESULTS: Between February 2005 and December 2015, 76 patients with genetically confirmed inherited myopathy were included. They were affected by Pompe disease or harbored mutations in RYR1, Collagen VI, LMNA, SEPN1, LAMA2 and MYH7 genes. Each myopathy had a specific pattern of affected muscles recognizable on mWB-MRI. This allowed us to create a novel decision algorithm for patients with rigid spine syndrome by segregating these signs. This algorithm was validated by five external evaluators on a cohort of seven patients with a diagnostic accuracy of 94.3% compared with the genetic diagnosis. CONCLUSION: We provide a novel decision algorithm based on muscle fat replacement graded on mWB-MRI that allows diagnosis and differentiation of inherited myopathies presenting with spinal rigidity. KEY POINTS: • Inherited myopathies are rare, diagnosis is challenging and genetic tests require specialized centres and often take years. • Inherited myopathies are often characterized by spinal rigidity. • Whole body magnetic resonance imaging is a unique tool to orientate the diagnosis of each inherited myopathy presenting with spinal rigidity. • Each inherited myopathy in this study has a specific pattern of affected muscles that orientate diagnosis. • A novel MRI-based algorithm, usable by every radiologist, can help the early diagnosis of these myopathies.

Early-onset stroke with moyamoya-like syndrome and extraneurological signs: a first reported paediatric series.

BACKGROUND: Moyamoya syndrome is characterised by an occlusion of the carotid terminations with the development of collateral vessels. Our objective is to describe a series of infants presenting early-onset moyamoya-like syndrome, which may constitute a distinct entity. METHODS: From a cohort of children with rare cerebral vascular pathologies, we studied eight infants (28 days-1 year) with early-onset moyamoya-like syndrome demonstrated by angiography. We retrospectively analysed the patterns on MRI and MRA, as well as all other available data. RESULTS: Median age at diagnosis was 7 months (IQR: 6-8) with arterial ischaemic stroke in the middle cerebral artery territory. All of the children experienced severe stroke recurrence within a median time of 11 months (IQR: 10-12), and all showed extraneurological symptoms. The anterior cerebral circulation was involved in all cases and the posterior circulation was involved in six. Two children died and all of the other children suffered permanent neurological deficits. CONCLUSIONS: The presence of extraneurological signs in cases of early-onset moyamoya syndrome is suggestive of a newly described systemic vasculopathy with predominantly cerebrovascular expression. Given its rapid progression marked by severe recurrent strokes and poor clinical outcome, early diagnosis could help in the decision to institute aggressive therapy.

Non-ischemic cerebral enhancing lesions secondary to endovascular aneurysm therapy: nickel allergy or foreign body reaction? Case series and review of the literature.

INTRODUCTION: Delayed onset of non-ischemic cerebral enhancing (NICE) lesions is a rare complication of intracranial aneurysms' endovascular therapy (EVT). The purpose of this study is to report this rare complication and its potential pathophysiology in a single-center case series and review the relevant literature. METHODS: After retrospective review of all patients managed by EVT at our institution from January 1, 2012 to December 31, 2014, 2 out of 374 patients (0.5 %) with such a complication were identified. Skin patch testing was performed with all endovascular devices used in the two patients and with the European baseline series, including nickel. All previously published cases in the English literature were reviewed based on exhaustive PubMed and Embase research. RESULTS: Patient no. 1 developed NICE lesions 1 month after balloon-assisted coiling of a ruptured anterior communicating artery aneurysm. Patient no. 2 developed NICE lesions 12 months (the longest delay reported to date for such a complication) after the treatment of a right carotid-ophthalmic aneurysm by loose coiling and flow diversion. Patient no. 2 demonstrated nickel skin reactivity, but none of the two patients presented allergic reaction to the devices used during interventions. CONCLUSIONS: Based on our observations and review of the literature, we hypothesize that delayed non-ischemic cerebral enhancing lesions after EVT are more likely related to foreign body emboli rather than nickel allergy. The two presented cases demonstrate the potential for recurrence and prolonged fluctuation of NICE lesions, warranting long-term follow-up for all patients presenting this complication.

Pre-surgical CT-assessment of neurogenic myositis ossificans of the hip and risk factors of recurrence: a series of 101 consecutive patients.

BACKGROUND: Neurogenic Myositis Ossificans (NMO) is a rare disabling pathology characterized by peri-articular heterotopic ossifications following severe peripheral or central nervous system injuries. It results in ankylosis and vessels or nerves compressions. Our study aimed to describe the pre-operative findings of patients with NMO of the hip using biphasic computerized tomography (CT). METHODS: Between 2006 and 2012, we retrospectively analyzed 101 consecutive patients with hip NMO. We analyzed all CTs and surgical reports following a standardized grid depicting the osteoma and its relations with joint capsule, vessels and nerves and bone mineralization. We studied surgical complications and recurrence during follow-up. Chi2-test and Fischer's test were performed to compare qualitative values with respectively normal and non-normal distribution. Quantitative values were analyzed with a one factor analysis of variance (ANOVA) test. Agreement between pre-surgical CT and surgical observations was evaluated with Cohen's kappa test. RESULTS: Correlation between pre-operative CT and surgical findings was excellent regarding relationships with vessels (0,82) and was good concerning relationships with sciatic nerves (0.62) and with joint capsule (0.68). Close contact or disruption of joint capsule (p = 0.005), joint space narrowing (p = 0.007) and bone demineralization (p < 0.001) were correlated with NMO recurrence. CONCLUSIONS: Biphasic enhanced-CT allows pre-operative assessment of NMO with good correlation to surgical observations and helps prevent surgical complications.

Rupture of the retrocorporeal artery: a rare cause of spontaneous spinal epidural haematoma.

A 22-year-old man presented with a sudden backache and paraplegia (ASIA = B). Magnetic resonance imaging showed an anterior pan-spinal epidural haematoma. Digital subtraction angiography was performed and ruled out an underlying vascular malformation but showed an active contrast media leakage into the T-4 ventral epidural space with a pattern of pseudo-aneurysm. A rupture of a T-4 retrocorporeal artery was considered as the aetiology, possibly caused by a haemorrhagic sub-adventitial dissection. Treatment consisted in the embolisation of both the pseudo-aneurysm and the parent artery with liquid acrylic glue, followed by neurosurgical decompression in emergency. The patient had totally recovered (ASIA = E) by the 10-month clinical follow-up.

Risks of presurgical embolization of feeding arteries in 137 intracranial meningeal tumors.

BACKGROUND: Embolization of extra-axial tumors has shown its effectiveness in reducing perisurgical blood loss. However, the complication rate of this procedure is poorly reported. We aimed to evaluate the rate of procedure-related complications and their risk factors. METHODS: From 1998 to 2011, 193 consecutive patients (141 females, 52 males; mean age = 52.9 years) were referred to our institution for presurgical embolization of an extra-axial tumor (meningiomas: n = 178; solitary fibrous tumors: n = 3; other: n = 12). Of 193 patients, 137 (71 %) underwent 141 embolizations (by microparticles: n = 133; by glue: n = 8). The remaining 56 patients (29 %) were not embolized due to unstable catheterization or dangerous anastomosis. Occurrence of neurological deficit was systematically assessed during and after embolization. The risk factors of procedure-related neurological complications were evaluated. RESULTS: Neither intratumoral hemorrhage nor procedure-related death was reported. Two of the 137 patients (1.5 %) had ischemic events with permanent neurological deficit after microparticles embolization. One patient had cortical blindness and one had hemiparesis. Both complications involved the vertebrobasilar system. The first patient had direct intratumoral anastomosis between the middle and the posterior meningeal arteries (PMA); the second one had reflux in the vertebral artery during particles injection in the PMA. Occurrence of ischemic complication was not related to the size of the microparticles. CONCLUSIONS: Though embolization of meningeal tumors is considered as a safe technique, serious neurological complications may occur. Opening of dangerous anastomosis or uncontrolled reflux caused two neurological complications (1.5 %). The size of the microparticles was not associated with the occurrence of neurological event.

Combined Angio-Seal™ and stenting rescue treatment in a case of iatrogenic common carotid artery dissection during direct puncture for ruptured intracranial aneurysm embolization: a technical note.

BACKGROUND AND IMPORTANCE: Direct puncture may offer an alternative access for embolization of intracranial aneurysms in patients presenting with tortuous vessels. Nevertheless, major complications such as compressive hematoma and arterial dissection can occur with this technique. CLINICAL PRESENTATION: A tight common carotid artery (CCA) dissection was seen secondary to direct puncture in a 72-year-old patient who presented with a ruptured anterior communicating artery (ACom) aneurysm. After regular coiling of the aneurysm and using a femoral approach, an Angio-Seal™ device (St Jude Medical, Saint Paul, MN, USA) was placed and a carotid wallstent (Stryker Neurovascular, Fremont, CA, USA) successfully deployed at the dissected CCA, which was followed by good clinical and angiographic outcomes. CONCLUSION: A combined rescue technique combining Angio-Seal insertion and stent deployment was safe and effective for managing iatrogenic carotid artery dissection.

Elevation of brain ADC (apparent diffusion coefficient) in HIV-associated neurocognitive disorders and evolution after treatment: A pilot study.

BACKGROUND: Thirty to 50% of HIV-infected patients develop HIV-Associated Neurocognitive Disorders (HAND) despite virological control. The previously published Neuro+3 study showed their neurocognitive status can be improved by intensifying antiviral therapy. Our study is a part of the Neuro3+ study and aims to study apparent diffusion coefficient (ADC) as a biomarker for neurological improvement. PATIENTS AND METHODS: We prospectively included 31 patients with HAND. They received therapy with better CNS Penetration Effectiveness (CPE) score with two-year follow-up. Cognitive status was assessed at day 0 (D0) and week 96 (W96) using Frascati 3-stage classification and Global Deficit Score (GDS). Brain MRI at D0 and W96 assessed morphological data (white matter hyperintensities, opportunistic infections, ischemic lesions, atrophy) and measured whole brain apparent diffusion coefficient (ADC). We compared their data with a control group of 20 healthy patients with similar ages and sex ratio. RESULTS: After ARV intensification, cognitive status was significantly improved: GDS (n = 1,4 vs 1,0 p = 0.01) and Frascati scale (2HAD/22MND/7ANI vs 1HAD/8MND/17ANI p = 0.001). Mean ADC was significantly higher in patients at inclusion than in controls (0.88 × 10-3 mm2/s ± 0.06 vs 0.81 × 10-3 mm2/s ± 0.04, p = 0.0001). ADC decreased after treatment (0.88 × 10-3 mm2/s ± 0.06 vs 0.85 × 10-3 mm2/s ± 0.06 (p = 0,04). In subgroup analysis, ADC significantly decreased in clinically improved patients (0.89 × 10-3 mm2/s ± 0.07 vs 0.85 × 10-3 mm2/s ± 0.07 (p = 0,03)) and did not significantly change in non-clinically improved patients (0.86 × 10-3 mm2/s ± 0.07 vs 0.84 × 10-3 mm2/s ± 0.07 (p = 0,31)). After treatment, there was no significant difference between patients and controls (0.85 × 10-3 mm2/s ± 0.06 vs 0.81 × 10-3 mm2/s ± 0.04, p = 0.17). CONCLUSION: Whole-brain ADC is a good biomarker of HIV-associated neurocognitive disorders. It is significantly increased in patients with HAND compared with controls and significantly decreases after treatment. It is all the more important to have a quantitative biomarker as conventional imaging does not contribute to the diagnosis.